Epidemiology of Moyamoya Angiopathy in Eastern India

Abstract

IntroductionMoyamoya angiopathy (MMA) is a chronic, progressive intracranial vasculopathy with variation in prevalence and clinical manifestations across different populations. This study was aimed to estimate the frequency of MMA as an etiology of stroke and its epidemiological features in the largest cohort of MMA patients in India.MethodA single-centered cross-sectional observational study over a period of 5 years (2016–2021) was undertaken among consecutive stroke and transient ischemic attack (TIA) patients to look for the presence of MMA angiographically. Each patient with angiographically proven MMA was further evaluated for demographic, clinical, and radiological characteristics.ResultsAmong 10,250 consecutive stroke and TIA patients (ischemic = 78%, hemorrhagic = 22%), frequency of MMA was 1.56% (n = 160); 15.3% among children. Female preponderance (Male:Female = 1:1.4) was noted among 160 MMA patients, with bimodal age distribution, first peak at 3–8 years, and a shorter second peak at 41–47 years. Childhood-onset MMA was seen in 75 (46.9%) with commonest initial neurological symptom of fixed-motor-weakness (44.0%), followed by TIA (26.7%); while 85 (53.1%) had adult-onset MMA with fixed-motor-weakness (50.6%) followed by headache (24.7%) as the predominant initial neurological symptom; seizure significantly higher in children (p < 0.001) and headache in adults (p = 0.012). Transient and fixed neurological manifestations constituted 87.5 and 69.4% respectively, of symptoms throughout the disease course. Cerebral infarction (45.0%) and TIA (21.9%) were the commonest types of MMA. On brain imaging, infarction was noted in 80.6%, hemorrhage in 11.3%, significantly higher among adults (p < 0.001). Cortical infarct and Gyral pattern were commoner in children (p = 0.004), subcortical infarcts in adults (p = 0.018). Frequent Suzuki staging observed was stage 4 (31.3%), followed by stage 3 (30.0%). Involvement of posterior circulation was detected in 55.6%, brain atrophy at the time of diagnosis was seen in 65.0%.ConclusionMMA is an important etiological consideration in patients with stroke, especially in children. It can present with a myriad of transient neurological symptoms, frequently overlooked, leading to delayed diagnosis, and contributing to socio-economic burden. Indian MMA showed aberrations in its gender predisposition, age distribution, frequency of familial cases, disease manifestation, and type of stroke, in comparison to its Japanese and Caucasian counterparts pointing to the inter- and intra-continent differences of MMA phenotype. Future development of the Indian MMA national registry is of essence.

Covid-19 vaccine (covishield) heralding Moyamoya angiopathy

“…Inflammatory state has been postulated to initiate and enhance progression of changes of MMA influencing the cytokine pathways. ( Das et al, 2019 , Das et al, 2020 , Das et al, 2021 , Das et al, 2021 , Das et al, 2021 , Das et al, 2022 , Das et al, 2022 ) We herein report two index case of young patient presenting with acute ischemic stroke and TIA finally diagnosed to be MMA, with neurological symptoms precipitated in close temporal association to COVISHIELD vaccination.…”

Headache Associated with Sexual Activity Unveiling Moyamoya Disease

“…Moyamoya angiopathy (MMA) is an intracranial vasculopathy characterized by bilateral progressive steno-occlusive changes in the intracranial portion of internal carotid arteries (ICA) and proximal portions of the anterior cerebral artery (ACA) and/or middle cerebral artery (MCA) with the development of compensatory abnormal vascular network at the base of the brain. [ 1 ] MMA can present with a wide range of neurological symptoms, which can be less infrequently subtle and may lead to a delayed diagnosis of MMA. [ 2 , 3 ] Headache in MMA is not uncommon, but frequently under-recognized.…”

“…Relevant investigations were conducted to rule out secondary causes of MMA. [ 1 ] The patient was counseled to assume a more passive position during sexual activity. He was prescribed oral naproxen (500 mg) for acute symptomatic therapy and planned for revascularization surgery.…”

“…The prevalence of headache in MMA varies with ethnicity and geographical distribution, as evidenced by its frequency of 50% in Europe, 21.6–25% in South Korea, and 36.8% in India. [ 1 , 2 , 3 , 4 , 5 , 6 ] Headache in MMA most commonly resembles migraine, either alone or combined with tension-type headache. [ 3 ] However, headache phenotypically mimicking headache associated with sexual activity in MMA is very uncommon.…”

“…[ 6 ] Das et al . [ 1 ] classified headache associated with MMA as a transient perfusion-dependent neurological symptom. These transient perfusion-dependent neurological symptoms in MMA often occur in close temporal association with the occurrence of precipitating factors, including heavy exercises and heightened emotional states, both of which may occur during sexual activity.…”